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Brain Research Jul 2020Gene therapy clinical trials for neurological disorders are ongoing using intrathecal injection of adeno-associated virus (AAV) vector directly into the cerebral spinal... (Review)
Review
Gene therapy clinical trials for neurological disorders are ongoing using intrathecal injection of adeno-associated virus (AAV) vector directly into the cerebral spinal fluid. Preliminary findings from these trials and results from extensive animal studies provides compelling data supporting the safety and benefit of intrathecal delivery of AAV vectors for inherited neurological disorders. Intrathecal delivery can be achieved by a lumbar puncture (LP) or intracisterna magna (ICM) injection, although ICM is not commonly used in clinical practice due to increased procedural risk. Few studies directly compared these delivery methods and there are limited reports on transduction of the PNS. To further test the utility of ICM or LP delivery for neuropathies, we performed a head to head comparison of AAV serotype 9 (AAV9) vectors expressing GFP injected into the cisterna magna or lumbar subarachnoid space in mice. We report that an intrathecal gene delivery of AAV9 in mice leads to stable transduction of neurons and glia in the brain and spinal cord and has a widespread distribution that includes components of the PNS. Vector expression was notably higher in select brain and PNS regions following ICM injection, while higher amounts of vector was found in the lower spinal cord and peripheral organs following LP injection. These findings support that intrathecal AAV9 delivery is a translationally relevant delivery method for inherited neuropathies.
Topics: Animals; Brain; Cisterna Magna; Dependovirus; Female; Gene Transfer Techniques; Genetic Therapy; Genetic Vectors; Injections, Spinal; Lumbar Vertebrae; Mice; Mice, Inbred C57BL; Motor Neurons; Neuroglia; Spinal Cord; Spinal Puncture; Transduction, Genetic
PubMed: 32289279
DOI: 10.1016/j.brainres.2020.146832 -
No Shinkei Geka. Neurological Surgery Mar 2021Hydrocephalus is caused by excessive accumulation of cerebrospinal fluid(CSF)in the ventricles or the skull. Unlike acute hydrocephalus presenting with elevated...
Hydrocephalus is caused by excessive accumulation of cerebrospinal fluid(CSF)in the ventricles or the skull. Unlike acute hydrocephalus presenting with elevated intracranial pressure, chronic hydrocephalus is called normal-pressure hydrocephalus(NPH). Because the CSF volume increases slowly, the brain compressively deforms without increasing intracranial pressure. NPH should be diagnosed and treated according to the following three categories: idiopathic NPH(iNPH), secondary NPH(sNPH), and congenital NPH(cNPH). The intracranial CSF distribution in iNPH differed from that in sNPH or cNPH. In iNPH, the Sylvian fissure and basal cistern were conspicuously enlarged, whereas the convexity subarachnoid space was severely decreased. CSF distribution in the subarachnoid space specific to iNPH is known as "disproportionately enlarged subarachnoid space hydrocephalus(DESH)," which might be due to direct CSF communication between the lateral ventricles and the basal cistern at the inferior choroidal point of the choroidal fissure. After shunt surgery in a patient with NPH, the lateral ventricles and Sylvian fissure shrank from top to bottom, while the convexity subarachnoid space expanded. In NPH, except for obstructive hydrocephalus, the flow void sign on spin-echo T2-weighted images is usually observed around the aqueduct, which reflects the increased CSF movement.
Topics: Cerebral Ventricles; Cerebrospinal Fluid Shunts; Humans; Hydrocephalus; Hydrocephalus, Normal Pressure; Magnetic Resonance Imaging; Membrane Proteins; Nerve Tissue Proteins; Subarachnoid Space
PubMed: 33762452
DOI: 10.11477/mf.1436204393 -
Skeletal Radiology May 2024
Topics: Humans; Arachnoiditis; Laminectomy; Ossification, Heterotopic
PubMed: 37851082
DOI: 10.1007/s00256-023-04465-7 -
Neurology India 2021Post-infective hydrocephalus (PIH) arises as a complication of any CNS infection, and can be either communicating or noncommunicating. (Review)
Review
BACKGROUND
Post-infective hydrocephalus (PIH) arises as a complication of any CNS infection, and can be either communicating or noncommunicating.
OBJECTIVE
The aim of this article is to study the various causes of PIH and its pathophysiology and treatment.
MATERIAL AND METHODS
The literature was searched for articles describing the causes of PIH.
RESULTS
Common causes of PIH are CNS tuberculosis (TB), neurocysticercosis, and perinatal or neonatal infection. TBM is most likely to result in hydrocephalus out of all these manifestations of CNS TB, and hydrocephalus is more likely to occur early in the course, typically 4-6 weeks after the onset of TBM, and is more common among children as compared to adults. A trial of medical management (antitubercular therapy, steroids, and decongestants) can be given to patients with communicating hydrocephalus. Ventriculoperitoneal shunt is the most employed method of CSF diversion in these patients. Though traditionally considered contraindicated, many recent studies have found ETV to be a reasonable option in patients with PIH. HCP in patients with neurocysticercosis can be associated with intraventricular cysts and racemose cysts in the basal subarachnoid cisterns. Surgical intervention is required either for cyst removal or CSF diversion. Endoscopic approaches can be used to remove the intraventricular cysts, which takes care of the HCP. PIH in infants can result either from antenatal infections (TORCH infections) or postnatal infections such as meningitis.
CONCLUSIONS
Management of PIH can be challenging. Management has to be individualized.
Topics: Adult; Child; Female; Humans; Hydrocephalus; Infant; Infant, Newborn; Neurocysticercosis; Pregnancy; Subarachnoid Space; Ventriculoperitoneal Shunt; Ventriculostomy
PubMed: 35102983
DOI: 10.4103/0028-3886.332273 -
Child's Nervous System : ChNS :... Oct 2023The purpose of this review article is to outline the natural history, pathogenesis, anatomic considerations and surgical decision-making in caring for patients with... (Review)
Review
PURPOSE
The purpose of this review article is to outline the natural history, pathogenesis, anatomic considerations and surgical decision-making in caring for patients with intracranial arachnoid cysts.
METHODS
A review of the literature for intracranial arachnoid cysts was performed using Embase, PubMed, and Web of Science databases, including review of the bibliographies of eligible articles and the author's own experience.
RESULTS
Among those reviewed, 59 relevant original articles were included as well as illustrative cases from the authors own experience.
CONCLUSIONS
Arachnoid cysts are congenital lesions characterized by split arachnoid membrane, thick collagen in the cyst wall, absent traversing trabecular processes within the cyst, and hyperplastic arachnoid cells in the cyst wall. The underlying etiology is not entirely known, and they occur in greater proportion in males and in greater incidence with various genetic conditions including Down syndrome, mucopolysaccharidosis, schizencephaly, neurofibromatosis, autosomal dominant polycystic kidney disease (ADPKD), acrocallosal syndrome, and Aicardi syndrome. Most intracranial arachnoid cysts are incidentally found and occur in the middle cranial fossa, with the remaining occurring in the cerebellopontine angle, suprasellar cistern, quadrigeminal cistern, convexity, and posterior fossa/cisterna magna. The current article outlines the natural history, prevalence, demographic factors, and treatment decisions in managing patients with intracranial arachnoid cysts.
Topics: Humans; Male; Arachnoid Cysts; Cisterna Magna; Head; Schizencephaly; Female
PubMed: 37466684
DOI: 10.1007/s00381-023-06066-0 -
Current Pain and Headache Reports Jan 2021To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF). (Review)
Review
PURPOSE OF REVIEW
To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF).
RECENT FINDING
CVF is a recently recognized cause of spontaneous intracranial hypotension (SIH), an important secondary headache, in which an aberrant connection is formed between the spinal subarachnoid space and an adjacent spinal epidural vein permitting unregulated loss of CSF into the circulatory system. CVFs often occur without a concurrent epidural fluid collection; therefore, CVF should be considered as a potential etiology for patients with SIH symptomatology but without an identifiable CSF leak. Imaging plays a critical role in the detection and localization of CVFs, with a number of imaging techniques and provocative maneuvers described in the literature to facilitate their localization for targeted and definitive treatment. Increasing awareness and improving the localization of CVFs can allow for improved outcomes in the SIH patient population. Future prospective studies are needed to determine the diagnostic performance of currently available imaging techniques as well as their ability to inform workup and guide treatment decisions.
Topics: Cerebrospinal Fluid Leak; Fistula; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Subarachnoid Space; Veins
PubMed: 33475890
DOI: 10.1007/s11916-020-00921-4 -
Continuum (Minneapolis, Minn.) Feb 2021This article highlights both common structural causes of myelopathy, such as spondylotic disease, and infrequent but treatable causes, such as syringomyelia, spinal cord... (Review)
Review
PURPOSE OF REVIEW
This article highlights both common structural causes of myelopathy, such as spondylotic disease, and infrequent but treatable causes, such as syringomyelia, spinal cord herniation, arachnoid cyst, arachnoid band and web, epidural lipomatosis, Hirayama disease, and arachnoiditis.
RECENT FINDINGS
Neuroimaging improvements and availability have uncovered many structural abnormalities in the spines and spinal cords of patients who were asymptomatic or minimally symptomatic. Recent published clinical series have improved our knowledge of the natural history of structural abnormalities and the risks of intervention versus conservative management.
SUMMARY
Myelopathy from a suspected structural cause is a common reason for neurologic consultation. Correlation between the history, examination, and imaging are especially important to determine whether intervention is necessary or conservative management is the best option.
Topics: Arachnoid Cysts; Arachnoiditis; Humans; Magnetic Resonance Imaging; Neuroimaging; Syringomyelia
PubMed: 33522741
DOI: 10.1212/CON.0000000000000975 -
Cellular and Molecular Life Sciences :... Mar 2021Cerebrospinal fluid (CSF) is produced by the choroid plexuses within the ventricles of the brain and circulates through the subarachnoid space of the skull and spinal... (Review)
Review
Cerebrospinal fluid (CSF) is produced by the choroid plexuses within the ventricles of the brain and circulates through the subarachnoid space of the skull and spinal column to provide buoyancy to and maintain fluid homeostasis of the brain and spinal cord. The question of how CSF drains from the subarachnoid space has long puzzled scientists and clinicians. For many decades, it was believed that arachnoid villi or granulations, outcroppings of arachnoid tissue that project into the dural venous sinuses, served as the major outflow route. However, this concept has been increasingly challenged in recent years, as physiological and imaging evidence from several species has accumulated showing that tracers injected into the CSF can instead be found within lymphatic vessels draining from the cranium and spine. With the recent high-profile rediscovery of meningeal lymphatic vessels located in the dura mater, another debate has emerged regarding the exact anatomical pathway(s) for CSF to reach the lymphatic system, with one side favoring direct efflux to the dural lymphatic vessels within the skull and spinal column and another side advocating for pathways along exiting cranial and spinal nerves. In this review, a summary of the historical and contemporary evidence for the different outflow pathways will be presented, allowing the reader to gain further perspective on the recent advances in the field. An improved understanding of this fundamental physiological process may lead to novel therapeutic approaches for a wide range of neurological conditions, including hydrocephalus, neurodegeneration and multiple sclerosis.
Topics: Animals; Arachnoid; Cerebrospinal Fluid; Cranial Nerves; Ethmoid Bone; Humans; Lymph Nodes; Lymphatic Vessels; Spine
PubMed: 33427948
DOI: 10.1007/s00018-020-03706-5 -
Injury Feb 2020Traumatic pneumorrhachis (PR) is a rare entity. There are only a few single cases published in English literature. In most of these cases PR was accidentally found...
INTRODUCTION
Traumatic pneumorrhachis (PR) is a rare entity. There are only a few single cases published in English literature. In most of these cases PR was accidentally found during CT-diagnostics and remained asymptomatic. The exact pathogenesis of traumatic PR has not been conclusively clarified. It is assumed, that a sudden increase in thoracic pressure causes air to escape the alveoli and migrates along the fasciae towards the spinal canal. In this study we reviewed the patients of our clinic for 13 years. Eight Patients with traumatic PR could be detected. This study represents the biggest account of traumatic PR in literature and gives a hint for the diagnostic and therapeutical regimen.
METHODS
We reviewed the radiological findings of our patients with thoracic trauma in the period from 2004 to 2016. We could detect eight patients with traumatic epidural PR and recorded any further injuries, therapies and outcome. Furthermore, a systematic literature review was carried out.
RESULTS
We found a total of eight patients suffering from traumatic PR. One of them hat a combination of epidural and subarachnoidal PR due to an open skull injury. Another of these patients got spondylodiscites nine months later at the level of the PR. In one Patient we could show the spontaneous reabsorption of the air in a CT-scan 4 days after trauma.
DISCUSSION
Traumatic PR remains a rare entity. It can be diagnosed with CT an MRT-scans. It needs no specific initial therapy besides the therapy of the underlying injuries. The prognosis of traumatic epidural PR is good and determined by the accompanying injuries. In cases of elevated paraclinical infection parameters one has to consider the development of spondylodiscitis in areas of PR.
Topics: Adult; Aged; Craniocerebral Trauma; Databases, Factual; Epidural Space; Female; Germany; Humans; Male; Middle Aged; Pneumorrhachis; Retrospective Studies; Spinal Canal; Spinal Diseases; Subarachnoid Space; Thoracic Injuries; Tomography, X-Ray Computed; Young Adult
PubMed: 31711655
DOI: 10.1016/j.injury.2019.10.075 -
Brain : a Journal of Neurology Sep 2020
Topics: Animals; Anti-Bacterial Agents; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Cisterna Magna; Drug Delivery Systems; Humans
PubMed: 32947618
DOI: 10.1093/brain/awaa280